Melanie Cole, MS (Host): Welcome to Better Edge, a
Northwestern Medicine podcast for physicians. I'm Melanie Cole. And joining me
today is Dr. Mariam Siddiqui. She's an Assistant Professor of Rheumatology at
Northwestern Medicine. She's here to tell us about connective tissue disease
and the intersection between rheumatology and dermatology.

Melanie Cole, MS: Dr. Siddiqui, it's such a pleasure
to have you with us. What a great topic. Connective tissue diseases often
present with both rheumatologic and dermatologic manifestations. Can you
explain the relationship between connective tissue disease disorders and the
skin? What are some of the common cutaneous manifestations observed in patients
with these conditions? And what are some of the common rheumatological and
dermatological symptoms that can overlap for connective tissue diseases?

Dr Mariam Siddiqui: Hi. Well, thank you for having
me. So yeah, a lot of questions right there. So, I treat primarily
rheumatologic diseases, so autoimmune connective tissue diseases. And these by
definition are systemic diseases, and they often affect multiple organs
including the skin. And I would say the skin is actually one of the more common
areas where we see manifestations of our diseases.

The common cutaneous manifestations that we see in our
patients kind of depends on the connective tissue disease that we're talking
about. For example, our lupus patients can present with things like rash and
photosensitivity. They can also have something called panniculitis, which is
like nodules under the skin. Conversely, our scleroderma patients can present
with things like calcinosis cutis, which is like calcium deposition under the
skin. So, we really see a wide variety of skin manifestations in our patients
depending on the disease.

Melanie Cole, MS: Well then, are there specific
connective tissue diseases that have a higher propensity for dermatologic
involvement? How does the presence of skin manifestations influence that
overall management and prognosis of these diseases?

Dr Mariam Siddiqui: While all of our autoimmune
connective tissue diseases can have some skin manifestations, I would say
things like systemic lupus erythematosus, cutaneous lupus is commonly seen.
There's a condition called dermatomyositis, which by definition involves the
skin, and it can be the only manifestation sometimes in our patients with
dermatomyositis. Morphea spectrum diseases, eosinophilic fasciitis, these are
autoimmune conditions that just affect the skin or primarily affect the skin.
And then, systemic vasculitis patients often have cutaneous manifestations as
well. But I'd say those are the ones that commonly we see with dermatologic
involvement.

The presence of skin manifestations, so it kind of depends
on the skin involvement in these patients. Some of these patients have really
extensive skin manifestations and that does affect my management because I'm
going to choose a systemic therapy in those cases. In cases where there's
milder skin involvement, we can get away in our clinic with using things like
topical therapies or UV therapy or things that don't necessarily suppress the
immune system like IVIg or hydroxychloroquine. We'll try using those instead of
something that's a little bit more immunosuppressive.

Melanie Cole, MS: Tell us how rheumatologists and
dermatologists collaborate to manage these patients with connective tissue
diseases. Discuss the importance of that interdisciplinary approach in
diagnosis and treatment.

Dr Mariam Siddiqui: So, I'd say rheumatology and
dermatology are very natural areas of collaboration, primarily because of what
we were talking about. So, rheumatic diseases often have dermatologic
manifestations and vice versa. Some dermatologic conditions often have
rheumatologic manifestations. I think the importance here of having both the disciplines
managing these patients grows out of this need to address really complicated
and refractory patients. I think that these combined clinics or
multidisciplinary clinics really grew out of this need. And as part of it,
we're able to provide very streamlined care for patients where our dermatology
colleagues assist with things like biopsies. They interpret and have a deeper
understanding of dermatopathology. They use topical therapies, UV therapies,
and even cosmetic therapies to guide our patients. And we as rheumatologists
are able to look at these patients and decide, is this just skin or does this
patient have systemic involvement of other organs? Is this joint pain actually
just wear and tear osteoarthritis or is this a part of their illness? And then,
we're able to help guide appropriate immunosuppression in these patients. So,
we really help in that area.

Melanie Cole, MS: Well then, tell us about
disease-specific programs at Northwestern Medicine and clinics that are joint
rheumatology and dermatology or include both specialists. Tell us about those.

Dr Mariam Siddiqui: So here at Northwestern, there's
many ways that we collaborate. So, some of the clinics have shared space and we
see patients at the same time as our dermatology colleagues and others are
physically separated, but coordinate very closely. So, for example, my clinic,
with Dr. Jennifer Shastry, is a connective tissue disease dermatology clinic.
We focus on things like cutaneous lupus, cutaneous vasculitis, morphea spectrum
disorders and dermatomyositis. There's another clinic with doctors Eric
Ruderman and Dr. Ahma Amin, and they have a shared space once weekly for
psoriatic arthritis patients. Here at Northwestern, we're also a scleroderma
center and get referrals from all over for our scleroderma specialists. They
don't have a shared space with the dermatologists, but they work very closely
with dermatologists and coordinate care.

I think in all of these areas, you basically have
dermatologists and rheumatologists who have experience treating our patients,
but also really enjoy collaborating with each other and treating these people.
And so, I think our multidisciplinary clinics, it's just the start of it in
terms of dermatology and rheumatology. I really think that these types of clinics
are standard of care in academic centers.

Melanie
Cole, MS: Well, they certainly are a more comprehensive approach. Now, Dr.
Siddiqui, can you share with us a specific complex patient case that you
collaborated with dermatology on to optimize care?

Dr Mariam
Siddiqui: Yeah. So, I think, in general we see a lot of refractory and very
complicated patients. But one of our earlier patients was a woman who had
actually come to see us with cutaneous lupus. And this involved her face and
she had something called lupus panniculitis of the face. And so, she had fat
atrophy, so she had indentations, and it really affected the way she looked and
the way she saw herself. And as a rheumatologist, I'm able to help with things
like immunosuppression to maybe help stop this process from occurring. But in
dermatology, they're really able to help. And Dr. Jennifer Shastry was really
great at guiding the patient not only with topicals, but once we were able to
stop the progression of this disease and we got everything stable with her
face, she was able to help guide the patient on things like autologous fat
transfers and more of the cosmetic side with using fillers to correct some of
this deformity that this disease had caused.

Melanie Cole, MS: What are some of the challenges or
complexities that arise when you're diagnosing and treating these connective
tissue diseases with prominent skin involvement? How do you navigate some of
those challenges?

Dr Mariam Siddiqui: So, I think one of the big things
is as rheumatologists, we often treat systemic disease, and a lot of it is
organ involvement, like heart, kidneys, brain involvement. With skin, it's a
little bit different. So, skin is on the outside. And so, patients have a huge
impact in terms of quality of life, their perception of self, their
self-esteem. A lot of that comes from what people can see on the outside. So, I
think one of the hardest things is we think about, "Oh, it's at least it's
not the heart and the kidneys that are involved." But skin is just as
important of an organ for the patient because it's what other people see of
them and it's how people can see that they're actually sick. So, I think
understanding what that means for the patient is important and it's sometimes
difficult. And knowing that sometimes we're going to have to use systemic
therapy and even really strong immunosuppressive therapy in order to help this
patient with their skin involvement.

Melanie Cole, MS: Dr. Siddiqui, as you're working
with these patients, are there any specific lifestyle modifications or
self-care practices that you advise your patients that can help improve the
quality of life for patients with these connective tissue diseases that overlap
with dermatologic symptoms?

Dr Mariam Siddiqui: Definitely. So in our cutaneous
lupus patients, one of the biggest things is that they're photosensitive. And a
lot of our patients are patients with skin of color, and so SPF is very
important. So, we talk to them about sunscreen. We talk to them about SPF, like
types of protection, physical and chemical barriers. We also talked to them
about clothing, that they can use wide-brimmed hats, things like that. So, a
lot of our education goes into like sun avoidance measures and how to protect
their skin. Because the sun, not only can it flare their systemic disease, it
can also cause worsening of their cutaneous disease. And it can also cause
hyperpigmentation in a lot of those patients that's really, really hard to
reverse. So, I would say probably the biggest lifestyle modification that we can
offer, is going to be sun protection.

Melanie Cole, MS: That's interesting. And lastly, Dr.
Siddiqui, are there any notable advancements, particularly regarding the
management of connective tissue diseases and their dermatologic manifestations
that rheumatologists other providers should be aware of?

Dr Mariam Siddiqui: Yes. So, one of the reasons I
enjoy rheumatology is that every year or so, there's a lot of new medications,
drugs that come down the pipeline. So, we're already using oral JAK inhibitors
in our clinic quite a bit for cases of dermatomyositis. But there's topical JAK
inhibitors that I don't think a lot of rheumatologists are reaching for, but
definitely our dermatology colleagues are. So, topical JAK inhibitors, they're
approved for things like psoriasis, for atopic dermatitis, vitiligo. Topical
tofacitinib is also used for things like periorbital discoid lupus. There's
topical high potency PDE4 inhibitors that are approved for things like
psoriasis.

Oftentimes our biologics for things like psoriatic arthritis
come from psoriasis patients. So, you know, there's these TYK2 inhibitors that
are being used or were approved in late 2022 for skin psoriasis and are looking
promising for psoriatic arthritis. Same type of thing for our cutaneous lupus
patients. There's drugs right now that are in phase II, phase III trials that
target plasmacytoid dendritic cells and they look really promising for our
cutaneous lupus patients. So, lots of drugs that are not only in the pipeline,
but are being used by our dermatology colleagues. And I think that they will
soon be used in rheumatology practices as well.

Melanie Cole, MS: What a great topic this was. Thank
you so much, doctor, for joining us today. To refer your patient or for more
information, please visit our website at
breakthroughsforphysicians.nm.org/rheum to get connected with one of our
providers. That wraps up this episode of Better Edge, a Northwestern Medicine
podcast for physicians. Please always remember to subscribe, rate and review
this podcast and all the other Northwestern Medicine podcasts. I'm Melanie
Cole.